[DaT-SCAN SPECT in the differential diagnosis of dementia with Lewy bodies and Alzheimer's disease]. / DaT-SCAN SPECT en el diagnóstico diferencial entre demencia con cuerpos de Lewy y enfermedad de Alzheimer.

INTRODUCTION: Dementia with Lewy bodies (DLB) is one of the main differential diagnosis of Alzheimer's disease (AD). In DLB there is 40-70% loss of striatal dopamine and the loss of dopaminergic cell is accompanied by loss of the dopamine transporter. The loss of dopaminergic neurons in DLB can be confirmed in vivo with I-FP-CIT (DaT-SCAN), a pre-synaptic dopamine transporter marker. There are no changes in DaT-scan in AD compared with controls. AIM. The use of DaT-SCAN for the differential diagnosis between AD and DLB. CASE REPORTS: We use the DaT-SCAN to study the nigrostriatal pathway in 6 patients with dementia and moderate parkinsonism. The Mini-Mental Test and the Unified Parkinson's Disease Rating Scale, motor part only (UPDRS), were performed. A SPECT scan was carried out 3 to 4 hours after administration of 185 MBq FP-CIT (ioflupane) a dopaminergic presynaptic ligand. With occipital cortex used as a radioactivity uptake reference, ratios for the caudate nucleus and the anterior and posterior putamen of both hemispheres were calculated. All scans were also rated by a simple visual method. All patients had dementia with moderate fluctuations in cognitive function, parkinsonian syndrome and hallucinations. DaT-SCAN was normal in 2 patients and pathological in 4. CONCLUSION: The sensitivity and specificity of the DLB criteria vary markedly. FP-CIT SPECT may be a new tool in the differential diagnosis between DLB and AD.

[Interictal basal brain SPECT versus interictal activated brain SPECT in temporal cryptogenic epilepsy]. / SPECT interictal basal frente a SPECT interictal activada en la epilepsia temporal criptogénica.

INTRODUCTION: Interictal epileptiform activity (IEA) is a common finding in temporal epileptic patients. The nature of the IEA is not entirely understood; it may be microseizures or just a negative phenomenon expressing brain inhibition mechanisms. In order to understand how brain metabolism is during interictal activity we studied a group of patients with SPECT HMPAO during basal condition no ictal activity - and when the same patients were having interictal activity. Temporal, thalamic and cerebellar perfusion were quantified by a digital method. PATIENTS AND METHODS: Two studies were performed in 24 epileptic patients: (9/15:M/F, 36,5 14,6 years). The MRI was normal or had minimal changes. Previous EEG had showed frontotemporal foci. The patients were studied by SPECT HMPAO performed with a STARCAM 3.200 equipment, the images being obtained by high resolution collimator with circular orbit 64 images, 30 s per image. The first study was performed when there was no anomaly in the EEG (basal study). In the second study (activated study) the radioligand was injected when the EEG showed at least 10 graphoelements (spike, polispikes, or sharp waves per EEG page). RESULTS: Thalamic, temporal and cerebellar perfusion decreased significantly in the activated state compared to the basal one (p < 0.001). The statistical relationship between these three locations was studied. There was a significant relationship (p < 0.001) between thalamic, temporal, and cerebellar perfusion, both in basal state and in the activated one. The correlation index was R2 > 0.9. CONCLUSIONS: For the cases studied, temporal epilepsy is associated to a significant decrease in thalamic, temporal and cerebellar perfusion in the basal state compared to the activated one. This finding may indicate that IEA expresses a post-ictal state.

[Fluctuations and dyskinesias as early L-dopa-induced motor complications in severe Parkinsonian's patients]. / Fluctuaciones y disquinesias como complicación precoz de la levodopaterapia en pacientes parkinsonianos graves.

Daily fluctuations of motor performance and dyskinesias in patients with Parkinson's disease (PD) treated with levodopa represent a difficult challenge to our understanding. We report 10 patients diagnosed of severe PD (Hoehn and Yahr: III-IV/V) treated with levodopa (range of dose: 750-900 mg/day) in single drug therapy since their diagnosis (mean time of levodopatherapy: 4.8 2.4 months, range: 3-6 months). All patients developed motor complications within weeks to months after initiating L-dopatherapy. Two patients received an intravenous apomorphine infusion (mean dose: 8.5 mg/day) during a mean time of 7.5 hours, but motor complications persisted during the infusion in spite of continuous dopaminergic stimulus. The degree of nigrostriatal damage (disease severity) seems to be a very important risk factor for the development of treatment-related motor complications.

[Primary orthostatic tremor: slow harmonic component as responsible of inestability]. / Temblor ortostático primario: componente armónico lento como responsable de la inestabilidad.

BACKGROUND: Orthostatic tremor (OT) is clinically defined as a tremor of the lower limbs and trunk on walking. It bears a significant functional impairement. Although the term orthostatic tremor was first used by Heilman in 1984, Pazzaglia et al had previously described some cases in 1970. Despite the fact that the pathophysiology of this entity is not fully known, the presence of a central oscillator is generally accepted as being responsible. A high frecuency tremor, between 13 and 18 Hz, constitutes an almost patognomonic finding, and treatment with clonazepam usually improves the symptoms. PATIENT AND METHOD: We present a patient who described his symptoms as "cramps" in lower limbs and trunk on standing up, which were relieved on walking or resting. RESULT: This clinical presentation together with a neurophysiological recording of the tremor showing an activity of lower frequency (8 Hz) combined with the usual higher frequency (16 Hz) and above all the clear amelioration of symptoms when treated with gabapentin, i.e. resolution of the low frequency tremor without changes in the 16 Hz tremor, were the peculiar features of this case which merits discussion. CONCLUSIONS: The slow component of the orthostatic tremor is crucial in this case. The improvement with gabapentin is explained by the disappearance of this slow c

[Motor impairment, in patients with severe Parkinson's disease, associated with dopaminergic hyperstimulation (entacapone)]. / Bloqueo motor, en pacientes parkinsonianos graves, en relación con sobrestimulación dopaminérgica (entacapone).

OBJECTIVE: [corrected] Entacapone was given to try to improve the motor complications in eight patients with Parkinson's disease (PD) treated chronically with levodopa, with daily severe motor fluctuations and dyskinesias. PATIENTS AND METHODS: We introduced entacapone (200 mg added to every dose of levodopa) to 8 parkinsonian patients (mean age: 68.25 +/- 2.3; range: 68-72; mean PD duration: 10.4 +/- 2.7 years) treated with oral levodopa, plus a dopa-decarboxylase inhibitor (mean dose: 706.25 +/- 2.3 mg/day; mean period of levodopa-treatment; 9 +/- 2.3 years). Dyskinesias were present in all patients (chorea: 8 patients; "off"--dystonia: 4; byphasic dyskinesias: 3). The type and duration (time "on" and "off") of fluctuations was categorized on the "on-off" charts drawn up by the patients or their relatives, and observation by the investigators after the introduction of entacapone. One patient, with severe impairment with entacapone, was evaluated (motor response) during i.v. apomorphine infusion (40 mg, during 3 hours). RESULTS: The combination of levodopa and entacapone was associated with a net increase in "off" time in all patients (from 5.8 +/- 1.2 h to 12.4 +/- 4.4 h) without change in the URPD. In the patient studied with i.v. apomorphine, "off" periods appeared during the infusion. CONCLUSION: These findings suggest that increased daily levodopa consumption may reduce striatal responsiveness to dopaminergic stimulation in severe parkinsonian patients. These data should be considered when planning the treatment strategy of complex parkinsonian patients.

[Falling backward: atypical sign of iodiopathic Parkison's disease. Use of intravenous apomorphine as a diagnostic test]. / Caídas hacia atrás: signo atípico para la enfermedad de Parkinson idiopática. Utilidad de la apomorfina intravenosa como test diagnóstico.

INTRODUCTION: The differential diagnosis of a parkinsonian syndrome is extensive and complex. In most cases, however, a detailed clinical examination will help to differentiate between idiopathic Parkinson's disease (IPD) and other causes of parkinsonism. PATIENTS AND METHODS: 10 patients with an average age of 65.1 years (range 60-70), of whom 6 were men and 4 women were referred to our department with a diagnosis of IPD and history of backwards falls. The average duration of disease was 4.7 years (range 4-7). All 10 patients had at presentation a severe akinetic-rigid syndrome with no other symptoms of IPD. I.v. apomorphine (APO) was administered to each of them at a rate of 8.9 mg/hr (range 15.3 mg/hr) for an average time of 5.4 hours (range: 3-10 hours). RESULTS: The improvement of the akinetic-rigid state in six of the patients during the i.v. infusion with APO helped to elicit signs that are atypical of IPD (ataxia and postural instability). Three of the patients did not respond to i.v. APO and one of them could not be evaluated, as he did not tolerate the infusion. Oral Dopa challenge or s/c APO bolus has not been as successful in helping to differentiate IPD from other parkinsonian syndromes. CONCLUSIONS: The prompt detection of postural instability or retropopulsion is necessary in order to diagnose atypical parkinsonian syndromes. In difficult cases, continuous i.v. APO will improve the akinetic-rigid state and facilitate the detection of atypical signs. The pharmacokinetic characteristics of APO, and the advantage of it being possible to administer the drug i.v. in a few hours increases the viability of this test.

Pituitary apoplexy: a transient benign presentation mimicking mild subarachnoid hemorrhage with negative angiography.

We report on a patient who presented with isolated transient headache as the only manifestation of pituitary apoplexy. A high index of suspicion and MRI led to the diagnosis. Copyright 1998 Lippincott Williams & Wilkins

Parkinsonian syndrome in childhood after sodium valproate administration.

Among the side effects attributed to sodium valproate administration, the production of a parkinsonian syndrome is very uncommon, particularly in children. We report a 12-year-old girl with secondary epilepsy; 7 days after the initiation of valproate therapy she developed parkinsonism that disappeared completely when valproate was replaced by carbamazepine. We discuss the possible role of alterations in GABAergic neurotransmission in the extrapyramidal syndrome that developed.

[Comparative study of magnetic resonance and evoked potentials in patients with clinically defined multiple sclerosis]. / Estudio comparativo entre resonancia magnética y potenciales evocados en pacientes con esclerosis múltiple clínicamente definida.

In an attempt to establish the efficacy of the most recent diagnostic tests--magnetic resonance (MR) imaging and multimodal evoked potentials (EP), 28 patients with clinically definite multiple sclerosis (MS) (1.a Poser's categories) were studied. The MR was positive in 26 (92%) patients; the EP were altered in 23 (82%)--the visual EP in 18 (64.2%), the auditory EP in 5 (17.8%), and the somatosensory EP in 15 (53.5%). Three patients with abnormal brain stem MR had normal EP. Two patients had normal MR and altered EP. There was not any patient with normal MR and EP. The MR was the most sensitive technique for the diagnosis of MS, but only during the 3 first years of evolution. After this study we consider the convenience to have both MR and EP positive to give a patient the diagnostic of clinically definite with paraclinic support (category 1.b of Poser).

[Sick sinus syndrome in children with a "healthy heart". Apropos of 2 cases with direct endocavitory tracing of the sino-atrial block]. / Maladie du sinus chez l'enfant à "coeur sain". A propos de deux cas avec enregistrement direct endocavitaire du bloc sino-auriculaire.

The sinus disease in children with "healthy hearts" is exceptional, and has never been documented by tracing of the sinus node. We are reporting two cases of two children, aged 4 and 14 years. An electrophysiological exploration with measurement of the direct activity of the sinus node, illustrates in one case the mechanism of sinus dysfunction. A review from the literature and our cases specifies some of the characteristics of this disease: 1) there are two forms: sporadic (case n. 1) 59 published cases, and familial (case n. 2) 28 published cases in 13 families; 2) the familial forms have a dominant autosomic transmission with variable penetration; 3) the disease may occur during the first days of life, suggesting a congenital origin (from the pathology findings, this disease may be one of the causes of the unexplained sudden death syndrome in infants; 4) association to atrio-ventricular conduction disorders and atrial and ventricular rhythm disorders; 5) frequent indication of stimulators, emphasizing the severity of this disease with a more severe course in sporadic forms (7 deaths in 59 cases).